Factors that Influence Health-Related Quality of Life in Patients with Primary Sclerosing Cholangitis.

BACKGROUND: Primary sclerosing cholangitis (PSC) is an incurable, cholestatic liver disease often coincident with inflammatory bowel disease (IBD). AIMS: To evaluate the impact of liver disease and IBD on health-related quality of life (HRQoL) in PSC. METHODS: A mixed-methods, cross-sectional study was performed at a tertiary center. Short Form-36 (SF-36) scores were compared between PSC, Canadian normative data, and disease controls. Disease-specific instruments scores [PBC-40, Short IBD questionnaire, Liver Disease Quality of Life Questionnaire (LDQOL)] were compared between PSC and disease controls. Multivariable regression identified factors independently associated with final SF-36 component scores. Qualitative evaluation of patient questionnaires was performed using a content analysis framework. RESULTS: One hundred and sixty-two surveys were completed (99 PSC, 26 primary biliary cirrhosis, 16 non-autoimmune cholestatic liver disease, and 21 IBD). PSC patients had significantly lower SF-36 scores than Canadian controls, but similar scores to disease controls. LDQOL most accurately predicted HRQoL. Factors negatively associated with physical HRQoL included shorter IBD duration, liver disease symptoms, and decompensated cirrhosis. Mental HRQoL was influenced by liver disease and IBD symptoms, pruritus, social isolation, and depression. Nearly 75 % expressed existential anxiety regarding disease progression and diminished life expectancy, with 25 % disclosing social isolation. CONCLUSIONS: Patients with PSC have significantly lower HRQoL than healthy controls. Both symptoms of IBD and chronic liver disease impact HRQoL in patients with PSC, which lead to significant psychologic burden that is expressed by existential anxieties and social isolation. A PSC-specific HRQoL tool is critical to adequately quantify the distinct impact of IBD and cholestatic liver disease.

IgG4 related disease - a retrospective descriptive study highlighting Canadian experiences in diagnosis and management.

BACKGROUND: Appreciating the utility of published diagnostic criteria for autoimmune pancreatitis, when compared to the characteristics of patients clinically managed as having disease, informs and refines ongoing clinical practice. METHODS: Comparative retrospective descriptive evaluation of patients with autoimmune pancreatitis including dedicated radiology review. RESULTS: 66 subjects with radiographic OR clinical features of autoimmune pancreatitis were initially identifiable (Male: n = 50), with 55 confirmed for evaluation. The most common presentation included pain (67%), weight loss (65%), and jaundice (62%). Diffuse enlargement of the pancreas was evident in 38%, whilst multifocal, focal, or atrophic changes were seen in 7%, 33% and 9% respectively. 13% had no pancreatic parenchymal involvement. Peripheral rim enhancement was seen in 23 patients (42%). Where discernible, disease was a) Sclerosing pancreatitis and cholangitis, n = 21; b) Sclerosing cholangitis, n = 9; c) Sclerosing pancreatitis, n = 4; d) Sclerosing pancreatitis and cholangitis with pancreatic pseudotumour, n = 7; e) Sclerosing cholangitis with hepatic pseudotumour, n = 3; f) Sclerosing pancreatitis with pancreatic pseudotumour, n = 1. 56% of the patients had systemic manifestations and the median serum IgG4 at diagnosis was 5.12 g/L. The Korean criteria identified most patients (82%) compared to HISORt (55%) or the Japan Pancreas Society (56%). The majority (HISORt 60%; Japan Pancreas Society 55%; Korean 58%) met diagnostic criterion by radiological findings and elevated serum IgG4. Treatment and response did not differ when stratified by diagnostic criteria. CONCLUSION: Our descriptive and retrospective dataset confirms that in non-expert practice settings, autoimmune pancreatitis scoring systems with a focus on radiology and serology capture most patients who are clinically felt to have disease.

Increased prevalence of and associated mortality with methicillin-resistant Staphylococcus aureus among hospitalized IBD patients.

OBJECTIVES: Methicillin-resistant Staphylococcus aureus (MRSA) infection has become increasingly prevalent in US hospitals, and the impact of MRSA on hospitalized inflammatory bowel disease (IBD) patients is unknown. METHODS: We used the Nationwide Inpatient Sample to identify admissions for IBD (n=116,842) between 1998 and 2004. We compared prevalence and in-hospital mortality of MRSA among IBD, non-IBD gastrointestinal (GI), and general medical inpatients. RESULTS: MRSA prevalence increased from 4.5/10,000 to 19.0/10,000 over the 7-year period (P<0.0001). After adjustment for confounders, IBD inpatients were at increased risk of MRSA compared with the non-IBD GI (adjusted odds ratio (aOR) 1.61; 95% confidence interval (CI): 1.33-1.96) and general medical (aOR 1.36; 95% CI: 1.11-1.66) groups. Of those with MRSA, catheter-related infections were specifically more common among IBD compared with non-IBD GI and general inpatients (28.8% vs. 11.0% and 8.5%, respectively, P<0.0002). Bowel surgery, parenteral nutrition, and health insurance were predictors of MRSA infection, but the first two became insignificant after controlling for length of stay (LOS). Compared with LOS < or = 7 days, MRSA was more likely among those hospitalized 8-21 days (aOR 7.40; 95% CI: 4.68-11.7) and >21 days (aOR 58.6; 95% CI: 36.0-95.3). MRSA infection was associated with sevenfold increase in mortality (aOR 7.61; 95% CI: 3.33-17.4). CONCLUSIONS: Hospitalized IBD patients are at increased risk of MRSA compared with non-IBD GI and general medical inpatients. Increased mortality in the IBD population associated with MRSA reinforces the importance of measures to prevent nosocomial infection and to reduce length of hospitalization.

Multiply relapsing hairy cell leukemia responsive to repeated courses of rituximab: a case report.

While cladribine is a highly effective therapy for patients with symptomatic hairy cell leukemia (HCL), up to 37% of patients ultimately relapse and incompletely responding patients relapse more frequently. Rituximab is a monoclonal antibody against CD20 that has been shown to be effective in patients with relapsed HCL. We present an unusual case of successful multiple re-treatments with rituximab in a patient with heavily pre-treated HCL and briefly review the relevant literature.

Contrast nephropathy post cardiac resynchronization therapy: an under-recognized complication with important morbidity.

OBJECTIVES: The aim of the study was to define the incidence of contrast nephropathy in patients undergoing cardiac resynchronization therapy (CRT). BACKGROUND: CRT is a promising new treatment for advanced heart failure. It is a technically demanding procedure with a recognized failure/complication rate. Contrast nephropathy is a well-recognized complication of coronary angiography/intervention, but has not been described following CRT. METHODS: We performed a retrospective chart review of patients who had undergone CRT at Mount Sinai Hospital, a tertiary referral center for heart failure management, to define the incidence of contrast nephropathy in patients undergoing CRT. Contrast nephropathy was defined as the occurrence of a 25% or greater increase in serum creatinine within 48 h after contrast administration. RESULTS: Sixty-eight patients underwent a total of seventy-three procedures between October 1st 2000 and December 31st 2003. Ten patients (14%) developed contrast nephropathy. Three of these patients (4%) required hemofiltration and one died. Patients with creatinine > or = 200 micromol/l (2.26 mg/dl) were more likely to develop contrast nephropathy than those with creatinine < 200 micromol/l (6/14 patients [43%] v 4/59 patients [7%], p<0.01). The mean length of hospital stay post-procedure in patients developing contrast nephropathy was 19+/-18 (SD) days versus 4+/-5 days for those patients with stable renal function (p<0.01). CONCLUSIONS: Contrast nephropathy is a frequent, but under-recognized complication of CRT with important morbidity/mortality. The extended hospital stay associated with contrast nephropathy has important clinical and health care implications. Patients and physicians need to be aware of this potential risk.

Cardiac resynchronization therapy: an option for inotrope-supported patients with end-stage heart failure?

BACKGROUND: Patients with refractory heart failure requiring inotropic support have a very poor prognosis. Cardiac resynchronization therapy (CRT) offers symptomatic and possibly a survival benefit for patients with stable chronic heart failure (CHF) and a prolonged QRS, but its role in the management of end-stage heart failure requiring inotropic support has not been evaluated. METHODS: We performed a retrospective observational study of patients undergoing CRT at our institution. RESULTS: We identified 10 patients who required inotropic support for refractory CHF and who underwent CRT while on intravenous inotropic agents. Patients had been in hospital for 30+/-29 days and had received inotropic support for 11+/-6 days prior to CRT. All patients were weaned from inotropic support (2+/-2 days post-CRT) and all patients survived to hospital discharge (12+/-13 days post-CRT). Furosemide dose fell from 160+/-38 mg on admission to 108+/-53 mg on discharge (p<0.01). Serum creatinine fell from 192+/-34 micromol/l prior to CRT to 160+/-37 micromol/l on discharge (p<0.05). Serum sodium was 131+/-4 mmol/l prior to CRT and remained low at 132+/-5 mmol/l on discharge. At short-term follow up (mean 47 days), all patients were alive; mean furosemide dose was 130+/-53 mg (p=0.056 versus pre-CRT). Serum creatinine was 157+/-36 micromol/l and serum sodium had increased to 138+/-6 mmol/l (p<0.05 and p<0.01, respectively, versus pre-CRT). CONCLUSION: CRT may offer a new therapeutic option for inotrope-supported CHF patients with a prolonged QRS.